The treatment and incidence of "butterfly disease", known in medical terms as Epidermolysis Bullosa, continues to be investigated. So, what exactly is butterfly disease and what are its symptoms?
Butterfly child disease is medically called Epidermolysis Bullosa. Water-filled lesions on the skin occur as a genetic disease. This disease, which is seen on average in 3 out of 100 thousand people, requires preventive treatment. On the other hand, the disease can negatively affect a person's life in later ages because it causes skin cancer.
WHAT IS BUTTERFLY CHILD'S DISEASE?
Epidermolysis bullosa is a genetic skin disease characterized by water-filled skin lesions called 'bulla'. It is popularly known as 'butterfly child disease'. Epidermolysis, skin laxity; bullosa are also two words used to mean water-filled bubble. The disease occurs as a result of genetic disruption of the adhesion of the epidermis and dermis. For nearly two decades, 13 major genes have been identified in the human body to cause this disease in most cases of epidermolysis bullosa. Both parents can be carriers of the diseased gene. In many countries, children suffering from this disease are called "Butterfly Children". Since the skin is as sensitive as a butterfly's wings, especially in children, the disease is compared with this term. In epidermolysis bullosa disease, the skin becomes extremely sensitive; In case of the slightest contact, friction and pressure, water bubbles can easily collect on the skin and skin integrity can be disrupted. In epidermolysis bullosa disease, water-filled lesions are not limited only to the outer skin, but can be seen in internal organs such as the mouth, esophagus, stomach, intestines and respiratory tract.
FREQUENCY OF THE DISEASE AND TYPES
A type of epidermolysis bullosa is seen in approximately 2-4 out of 100,000 people. The disease is seen equally regardless of race, color and gender. There are three main types and side types of the disease:
1. Epidermolysis Bullosa Simplex: It usually begins to appear with water collection at birth or immediately after. W In a local and mild type called Ebler Cockayne, the blisters extend beyond the hands and feet. In some side types of epidermolysis bullosa simplex, blisters form in large parts of the body.
2. Epidermolysis Bullosa Junctional: This type of the disease is generally severe and severe. It can cause life-threatening dehydration (liquid loss) and complicated infections with large blisters and ulcerated lesions on the face, trunk and legs.
3. Epidermolysis Bullosa Dystrophic: In some dominant and slightly recessive types, it can be seen only on the hands, feet, elbows and knees. Soft tissue, especially the esophagus, may be affected. In severe recessive types, it can cause blisters in large areas of the body, nail loss, growth retardation, eye inflammation, tooth decay, and adhesions on the fingers. The risk of developing a skin cancer called 'Sguamos cell carcinoma' is high.
DIAGNOSIS OF THE DISEASE
It is revealed as a result of a skin biopsy taken along with symptoms and findings. Thanks to today's medical examinations, genes that carry the disease can be detected from patients and family members through genetic analysis. Early diagnosis of the disease is possible with amniocentesis in the 10th week of pregnancy.
BUTTERFLY DISEASE TREATMENT
Mild types of epidermolysis bullosa may not require long treatment. However, efforts should be made to prevent blister formation and infection of blisters. In moderate and severe cases, there may be many complications. Psychological support along with protection and care of the skin and soft tissue may be required. There is no definitive treatment for Epidermolysis bullosa yet. Those infected with the disease die during childhood as a result of fluid electrolyte losses, complicated severe infections and multiorgan failure. Even in cases where they do not succumb to the disease during childhood, they die in their 20s or 30s due to contracting 'squamous cell carcinoma', a rapidly spreading skin cancer.
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